The records of 21 children with neuroblastoma presenting with spinal cord compression, encountered over 17 years, were reviewed. Thirteen patients (61%) survive, free of neuroblastoma, at intervals ranging from nine months to 192 months (median, 78 months) from the time of diagnosis. The explanation for this relatively high survival rate was sought in an analysis of the cases which took into account age, site, extent of disease, and histology. The most significant features to emerge were the unusually high proportion of children under 12 months of age at presentation (11 of 21) most of whom survive (9 of 11) and the low incidence (3 of 21) of detectable at the time of diagnosis. The absence of a paraspinal mass was an unfavourable prognostic features (1 of 6 survives) whilst if a paraspinal mass was present, its anatomical level did not influence survival. In particular, children with retroperitoneal tumors fared no worse (survival, 6 of 7) than those with primary tumors at other sites (survival, 6 of 8). Morbidity was high (6 of 13), principally in infants with spinal cord compression from birth. Survival was also related to the histologic maturity of the tumor, even in the presence of metastases. Recommendations for management are made.
43 cases of intracranial ependymomas in children 0–13 years of age have been reviewed with attention given to various clinical aspects, including location, age, duration of history, signs and symptoms, and plain skull film abnormalities. 39 of the patients were treated by intracranial surgical procedures and 30 of these also had radiation therapy. Three patients had needle biopsy only and one died without treatment. The survival rate was below that anticipated and in part felt to be related to the frequent occurrence of malignant tumors in this series.
THE surgical treatment of myelo-meningoceles is a controversial subject and it is not proposed in this short paper to discuss the indications for operation or the time at which it should be carried out. Once it has been decided that the lesion should be explored and the sac excised, it is essential to achieve complete FIG. 6y5.-'l'he circular defect following excision ,of a largc myelo-meningocelc can be resolved into two triangles.
Summary. The families of 207 index patients treated for spinal dysraphism at The Hospital for Sick Children were studied to discover whether the condition was aetiologically related to the classical neural tube malformations-spina bifida cystica and anencephaly. The index patients had all had a tethered conus medullaris and one or more of a variety of anomalies of the spinal cord, vertebrae, or skin overlying the vertebral column.Of 364 sibs of index patients, 9 had anencephaly and 6 spina bifida cystica, a proproportion of 4.12%. This approximates to the proportion of sibs affected by neural tube malformations in the London region when the index patients themselves have spina bifida or anencephaly.It is, therefore, appropriate that the mothers of children with spinal dysraphism should be offered prenatal screening for neural tube malformations.
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